Treatment and survival in children with pulmonary arterial hypertension: the UK Pulmonary Hypertension Service for Children 2001-2006.
OBJECTIVE
A retrospective study of the UK Pulmonary Hypertension Service for Children for the first 5-year period of its existence.


METHODS
Records of 216 children with idiopathic pulmonary arterial hypertension (IPAH) and associated pulmonary arterial hypertension (APAH) were reviewed. Kaplan-Meier survival curves were constructed for different diagnostic groups and for different therapies.


RESULTS
At cardiac catheterisation only 7.4% of those with IPAH and 6% of those with APAH responded positively to vasodilator testing and so were treated with nifedipine. Others needing treatment were given continuous intravenous epoprostenol, bosentan or sildenafil singly or in combination. For IPAH survival rates were 85.6%, 79.9% and 71.9% at 1, 3 and 5 years, respectively, compared with a survival time of less than a year in historical untreated controls. A combination of intravenous epoprostenol with either bosentan or sildenafil, or both, appeared to achieve the best outcome. Six children underwent lung transplantation. In APAH survival rates were 92.3%, 83.8% and 56.9% at 1, 3 and 5 years, respectively, postoperative congenital heart disease with severe pulmonary hypertension having the worst outcome.


CONCLUSIONS
New pulmonary hypertension-specific medicines have improved survival in children as in adults. Outcome in this series compares favourably with international outcome data.
Haworth
S G
SG
UCL Institute of Child Health, University College London, 30 Guilford Street, London, UK.
Hislop
A A
AA
D000293:Adolescent; D000077300:Bosentan; D016022:Case-Control Studies; D002648:Child; D002675:Child, Preschool; D011464:Epoprostenol / Q000627:therapeutic use; D005260:Female; D006330:Heart Defects, Congenital / Q000150:complications / Q000401:mortality / Q000601:surgery; D006801:Humans; D006976:Hypertension, Pulmonary / Q000188:drug therapy* / Q000401:mortality* / Q000601:surgery; D007223:Infant; D053208:Kaplan-Meier Estimate; D016040:Lung Transplantation; D008297:Male; D009543:Nifedipine / Q000627:therapeutic use; D010879:Piperazines / Q000627:therapeutic use; D011687:Purines / Q000627:therapeutic use; D012189:Retrospective Studies; D000068677:Sildenafil Citrate; D013449:Sulfonamides / Q000627:therapeutic use; D013450:Sulfones / Q000627:therapeutic use; D015996:Survival Rate; D006113:United Kingdom; D014665:Vasodilator Agents / Q000627:therapeutic use*
D010879:Piperazines; D011687:Purines; D013449:Sulfonamides; D013450:Sulfones; D014665:Vasodilator Agents; D000068677:Sildenafil Citrate; D011464:Epoprostenol; D009543:Nifedipine; D000077300:Bosentan